Article
Application of CRISS score, revised CRISS score and RCID score in patients with Juvenile systemic sclerosis
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Authors
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Jens Klotsche
- German Rheumatism Research Center, Berlin
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Ivan Foeldvari
- Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
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Kathryn Torok
- University of Pittsburgh, Pittsburgh
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Francesco Del Gado
- University of Leeds, Leeds
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Daniel E. Furst
- University of California, Los Angeles
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Ozgur Kasapcopur
- jSSc collaborative group, Hamburg
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Amra Adrovic
- jSSc collaborative group, Hamburg
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Brian Feldman
- jSSc collaborative group, Hamburg
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Maria Teresa Terreri
- jSSc collaborative group, Hamburg
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Ana Paula Sakamoto
- jSSc collaborative group, Hamburg
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Flavio Sztajnbok
- jSSc collaborative group, Hamburg
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Jordi Anton
- jSSc collaborative group, Hamburg
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Maria Katsicas
- jSSc collaborative group, Hamburg
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Valda Stanevicha
- jSSc collaborative group, Hamburg
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Simone Appenzeller
- jSSc collaborative group, Hamburg
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Tadey Avcin
- jSSc collaborative group, Hamburg
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Sindhu Johnson
- jSSc collaborative group, Hamburg
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Mikhail Kostik
- jSSc collaborative group, Hamburg
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Hana Malcova
- jSSc collaborative group, Hamburg
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Edoardo Marrani
- jSSc collaborative group, Hamburg
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W.-Alberto Sifuentes-Giraldo
- jSSc collaborative group, Hamburg
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Raju Khubchandani
- jSSc collaborative group, Hamburg
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Dana Nemcova
- jSSc collaborative group, Hamburg
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Maria Jose Santos
- jSSc collaborative group, Hamburg
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Dieneke Schonenberg
- jSSc collaborative group, Hamburg
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Christina Battagliotti
- jSSc collaborative group, Hamburg
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Lillemor Berntson
- jSSc collaborative group, Hamburg
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Blanca Bica
- jSSc collaborative group, Hamburg
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Juergen Brunner
- jSSc collaborative group, Hamburg
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Despina Eleftheriou
- jSSc collaborative group, Hamburg
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Liora Harel
- jSSc collaborative group, Hamburg
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Gerd Horneff
- jSSc collaborative group, Hamburg
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Tilmann Kallinich
- jSSc collaborative group, Hamburg
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Thomas Lehman
- jSSc collaborative group, Hamburg
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Kirsten Minden
- jSSc collaborative group, Hamburg
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Monika Moll
- jSSc collaborative group, Hamburg
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Susan Nielsen
- jSSc collaborative group, Hamburg
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Anjali Patwardhan
- jSSc collaborative group, Hamburg
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Vanessa Smith
- jSSc collaborative group, Hamburg
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Nicola Helmus
- Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
| Published: | August 30, 2023 |
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Outline
Text
Introduction: Juvenile systemic sclerosis (jSSc) is a rare disease in childhood. To date, no composite response index exists to assess treatment effect in jSSc patients. ACR CRISS score (probability of improvement ranging from 0 to 1 based on mRSS, FVC%, PtGA, MDGA and HAQ-DI) and revised ACR CRISS (rCRISS, proportion of patients who improve in ≥3/5 ACR CRISS core items by a certain percentage, e.g. 30%, except 5% for FVC) were developed by experts in the field as outcome measures in adult patients with SSc. In addition, the Ranked Composite Important Difference (RCID) score was recently introduced as anchor to the ACR CRISS.
Methods: Data from the international jSSc inceptions cohort were used for this analysis. The ACR CRISS, rCRISS and RCID were calculated between baseline and 12-months follow-up according to the scoring algorithms. Missing values in the core items were estimated by multiple imputation by chained equations. Here we aimed to determine the value of the response measures to detect clinically change defined by the anchor questions about change (much better or little better versus almost the same, little worse or much worse) in patients overall health due to scleroderma since the last visit provided by the treating physicians and parents or patients (aged >12 years).
Results: We included 95 jSSc patients with diffuse cutaneous subtype with available baseline and 12-months visit. Seventy-nine percent were female, the mean age at enrollment was 13.0 (3.8) and the mean disease duration was 3.1 (2.8) years. Among 95 patients, 57% were treated with steroids, 47% with methotrexate, 27% with MMF and 3% with a biological at baseline. ACR CRISS showed a ceiling effect (>.998) in 51% and a floor effect (<0.005) in 26% of patients. Patients who reported at least moderate improvement had a median ACR CRISS of 0.99 and in mean 2.6 (1.3) core items that improved by ≥20% from baseline to 12-months follow-up. The rCRISS 20/30/50 responses were 59%/49%/33% in patients who reported improvement (table 1 [Tab. 1]) and 25%/25%/8% in patients with worsening. The RCID was approximately normal distributed (mean 20.7, SD 43.4). Mean (SD) RCID for patients who reported worsening was -10.5 (38.6) vs RCID of 20.7 (45.2) for patients who reported improvement. RCID scores for physician reported anchors of worsening or improvement were 6.5 (44.2) and 18 (45.4), respectively. The concordance between a positive RCID score and rCRISS 20/30 was moderate (rCRISS 20 and RCID, 43%, kappa=0.43; rCRISS 30 and RCID, 38%, kappa=0.36).
Conclusion: Our data confirmed the presence of a ceiling and floor effect of ACR CRISS as shown in studies of adult SSc patients. The CRISS, rCRISS and RCID response distinguished between patients who rated their disease course since last visit as worsened or improved. Future studies should focus on the determination of specific pediatric weights for the CRISS and RCID components rather than extrapolation from adult SSc. In general, the RCID offers a meaningful tool in order to determine response to therapy in future clinical trials in jSSc patients.
